Neonatal hypoglycemia: unraveling a mystery.
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Biomedical subjects
Publications and source records attributed to W S Ball.
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PURPOSE: We describe imaging features that are clues to the diagnosis of atretic cephaloceles and discuss clinical findings and a possible mechanism by which these lesions develop. METHODS: Eight children (five girls and three boys) ranging in age from 1 day to 3 years 4 months with midline subscalp lesions underwent radiologic examination with CT or MR imaging. In all cases, the lesions were surgically excised and subjected to pathologic examination. Imaging studies and medical records were reviewed retrospectively. RESULTS: Six of eight children had vertical embryonic positioning of the straight sinus with a prominent superior cerebellar cistern. A "spinning-top" configuration of the tentorial incisura, a "cigar-shaped" CSF tract within the interhemispheric fissure, fenestration of the superior sagittal sinus, and "peaking" of the tentorium were associated findings helpful in making this diagnosis. Two of the eight children had findings indistinguishable from focal dermoid, six were developmentally normal, one had mild motor delay, and one died at the age of 3 years. Pathologic examination revealed glial, meningeal (arachnoid), fibrous, and dermal elements. CONCLUSION: Characteristic findings on MR images and CT scans provide clues to the diagnosis of atretic cephalocele. However, even in the presence of abnormal imaging findings, these children may be developmentally normal.
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"Trilateral retinoblastoma" (TRB) is the association of a midline intracranial tumor with familial bilateral retinoblastoma. Classically, the intracranial tumor is a pineal region tumor (pineoblastoma) with histology similar to retinoblastoma. We present a 7-month-old child with bilateral orbital retinoblastoma with a third tumor arising in the chiasmatic cistern. The presentation and prognosis of patients with this association differ from those children with "classical" TRB. The presentation, treatment, and outcome are described, with a review of the literature.
Parry-Romberg syndrome is a poorly--understood disorder characterized by progressive hemifacial atrophy involving the skin, soft tissue, and bone. Involvement of the central nervous system with impairment in neurologic function occurs infrequently. We describe a child with this syndrome in whom central nervous system involvement, documented on serial MRI, played a prominent role. We have attempted to correlate the clinical course with the radiologic findings, and to determine the impact of prednisone and methotrexate on the intracranial lesions.
OBJECTIVE: The purpose of this study was to evaluate the safety and efficacy of sedating pediatric outpatients in a radiology department using a structured sedation program modeled after the guidelines of the American Academy of Pediatrics. MATERIALS AND METHODS: Medical records of 6006 pediatric outpatients who underwent sedation for imaging studies under the guidelines of a structured sedation program were retrospectively reviewed for safety and efficacy of sedation, complications related to sedation, and rate of successful sedation. RESULTS: No cases of cardiovascular or respiratory arrest or fatalities occurred. Four children who required overnight hospitalization because of CNS depression had no sequelae. Forty-eight patients experienced transient respiratory depression that proved responsive to supplemental oxygen. Delayed complications were found in 29 children, none of whom required hospitalization. The rate of sedation failure was approximately 1%. CONCLUSION: Sedation of children can be done in a safe and highly efficacious manner in a hospital radiology department using a structured sedation program modeled after the guidelines of the American Academy of Pediatrics.
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We report preliminary experience using single-voxel, proton MR spectroscopy (MRS) employing small voxels of interest, in combination with short and long echo-time protocols, for the assessment of primary intracranial tumors in children. We examined 23 children with primary intracranial tumors detected by MRI, and 32 controls with similar ages, using MRS on a 1.5 T system. Localized single-voxel (3.7 +/- 1.3 cc) proton spectra were obtained with short-echo (2,000/18), stimulated-echo (STEAM) and long-echo (2,000/270) spin-echo (PRESS) protocols. All spectra were evaluated qualitatively; 10 tumor and 19 control spectra were processed for peak area quantification. Small voxels of interest were able to account for tissue heterogeneity. Combined acquisition of short- and long-echo spectra increased the number of detectable metabolites. The solid portion of all tumors exhibited reduced N-acetyl-aspartate (NAA), strong contribution from cholines (Cho) and inositols or glycine, minimal presence of total creatine (tCr), enhanced broad mobile lipid resonances and accumulated lactate. Calculated selected metabolite ratios of Cho/tCr and Cho/NAA were substantially increased from control values. The cystic portions of the masses showed only lipid and lactate peaks.
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The case of a patient with sickle cell disease is presented in which neuropsychological and magnetic resonance imaging studies were completed prior to and after a right hemispheric stroke. The contribution of a new MR perfusion technique in understanding the neurological complications in this patient is discussed. This case illustrates the complex pathophysiology of neuropsychological deficits in SCD and underscores the need to develop models that better reflect this complexity.
Bone marrow transplantation (BMT) is the only curative therapy available for hemoglobinopathies. BMT was performed on a young child with sickle cell anemia (SCA) after approximately 9 months of transfusion therapy following her initial stroke. The patient received a matched sibling donor (sickle trait) BMT. The conditioning regimen consisted of busulfan 4 mg/kg/day x 4, cyclophosphamide 50 mg/kg/day x 4. Graft vs. host disease prophylaxis was daily cyclosporine for 6 months. There were no significant complications during BMT. Engraftment occurred on day +17 and the patient was transfusion independent since day +45. Pre-BMT cerebral arteriography showed multiple stenotic cerebral vessels and a moya-moya pattern. Perfusion MRI demonstrated reduced capillary perfusion. Approximately 170 days after BMT the patient experienced episodes of transient left-sided weakness and speech problems. Neuroimaging revealed progression of large vessel pathology by angiography despite significant improvement in cortical perfusion (MR perfusion scan). Molecular analysis by PCR and DNA fingerprinting confirmed absence of mixed mosaicism. Rheologic evaluation showed normal corrected bulk viscosity. It is possible that progression of large vessel pathology and return of clinical symptoms in the face of normal rheologic parameters may be due to worsening of the already damaged cerebral vessels by the BMT conditioning regimen. Further evaluations of patients with SCA undergoing BMT after a stroke are needed to answer this question.
We describe the clinical manifestations, associated abnormalities, MRI appearances and pathologic significance of a case of hypophyseal duplication. A 16-year-old girl presented with delayed sexual development and history of midline craniofacial anomalies. MRI revealed paired infundibula extending inferiorly to two small pituitary glands, a midline hypothalamic mass, and a midline cleft in the basisphenoid. Twelve cases of pituitary duplication have previously been described. The suggested pathogenesis is duplication of the prechordal plate and anterior end of the notochord during early embryologic development.
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Two patients with congenital dermoids of the eustachian tube presented with recurrent otitis media and chronic otorrhea resistant to antimicrobial therapy. CT demonstrated fat density, homogeneous lesions, filling and expanding the eustachian tube. On MR, signal from the lesions was consistent with fat, and the relationship with the internal carotid artery was better delineated than by CT. Microscopically, the masses consisted of a conglomeration of ectodermal and mesodermal elements.
We describe the MR appearance of kernicterus (bilirubin encephalopathy). T2-weighted images show high signal bilaterally in the globus pallidus, a known area of cerebral deposition of bilirubin.